Pediatric Movement Disorders Center

Pediatric Movement Disorders

Dystonia: Why you should see a Specialist at the Pediatric Movement Disorders Center

What is dystonia?

Catherine Mazzola, MD

Dystonia is a movement disorder characterized by sustained muscle contractions producing twisting, repetitive, and patterned movements or abnormal postures. Dystonia in children is usually caused by a genetic, metabolic, or perinatal (around the time of birth) cause. In rare cases, dystonia may be caused by a stroke or bleed in the brain. Dystonia is among the most commonly observed movement disorders in children with cerebral palsy. It is classified on the basis of cause, age at onset of symptoms, and distribution of affected body parts.

The etiology or cause of pediatric dystonia is quite variable. There are many different genetic syndromes and several causes of symptomatic syndromes. Dystonia can be secondary to virtually any damaging process that affects the brain, and particularly the basal ganglia.

Treatment for most forms of dystonia is symptomatic and includes drugs (systemic or focal treatments, such as botulinum toxin) and surgical procedures. There are several medications including anticholinergic, dopamine-blocking and depleting agents, baclofen, and benzodiazepines. In patients with dopamine synthesis defects L-dopa treatment may be very useful. Botulinum toxin (Botox) treatment may be helpful in controlling the most disabling symptoms of localized or focal dystonia. Intrathecal baclofen therapy (ITB), delivered through an implantable pump, may be very effective. In some cases, ITB delivers baclofen directly into the spinal fluid of the spinal canal or the ventricles of the brain. Long-term electrical stimulation of the globus pallidum appears to be especially successful in children suffering from generalized dystonia.

Baclofen Pumps

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